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Background: In order to successfully implement the international clinical care guidelines for Duchenne muscular dystrophy (DMD) in the Netherlands, it is essential to know what barriers are experienced by healthcare practitioners regarding guideline adherence and organization of care. In the Netherlands, academic medical centers provide follow up visits and work together with peripheral hospitals, rehabilitation centers, centers for home ventilation and primary care centers for treatment. Objective: To investigate perceived barriers to international clinical DMD guideline adherence and identify potential areas of improvement for implementation in the Dutch ‘shared care’ organization. Methods: Semi-structured in-depth interviews with healthcare practitioners of academic medical hospitals and questionnaires for healthcare practitioners of rehabilitation centers, based on the framework of Cabana. Results: The analyses identified 4 barriers for non-adherence to the DMD guideline: (i) lack of familiarity/awareness, (ii) lack of agreement with specific guideline, (iii) lack of outcome expectancy, (iv) external barriers. Conclusions: A heterogeneous set of barriers is present. Therefore, a multifaceted intervention strategy is proposed to overcome these barriers, including a clear division of roles, allowing for local (Dutch) adaptations per specialism by local consensus groups, and the facilitation of easy communication with experts/opinion leaders as well as between care professionals.

Background Regaining gait capacity is an important rehabilitation goal post stroke. Compared to clinically available robotic gait trainers, robots with an assist-as-needed approach and multiple degrees of freedom (AANmDOF) are expected to support motor learning, and might improve the post-stroke gait pattern. However, their benefits compared to conventional gait training have not yet been shown in a randomized controlled trial (RCT). The aim of this two-center, assessor-blinded, RCT was to compare the effect of AANmDOF robotic to conventional training on the gait pattern and functional gait tasks during post-stroke inpatient rehabilitation. Methods Thirty-four participants with unilateral, supratentorial stroke were enrolled (mDOF robotic (combination of training in LOPES-II and conventional gait training) or conventional gait training (30 min, 3–5 times a week), focused on pre-defined training goals. Randomization and allocation to training group were carried out by an independent researcher. External mechanical work (WEXT), spatiotemporal gait parameters, gait kinematics related to pre-defined training goals, and functional gait tasks were assessed before training (T0), after training (T1), and at 4-months follow-up (T2). Results Two participants, one in each group, were excluded from analysis because of discontinued participation after T0, leaving 32 participants (AANmDOF robotic n = 17; conventional n = 15) for intention-to-treat analysis. In both groups, WEXT had decreased at T1 and had become similar to baseline at T2, while gait speed had increased at both assessments. In both groups, most spatiotemporal gait parameters and functional gait tasks had improved at T1 and T2. Except for step width (T0–T1) and paretic step length (T0–T2), there were no significant group differences at T1 or T2 compared to T0. In participants with a pre-defined goal aimed at foot clearance, paretic knee flexion improved more in the AANmDOF robotic group compared to the conventional group (T0–T2). Conclusions Generally, AANmDOF robotic training was not superior to conventional training for improving gait pattern in subacute stroke survivors. Both groups improved their mechanical gait efficiency. Yet, AANmDOF robotic training might be more effective to improve specific post-stroke gait abnormalities such as reduced knee flexion during swing. Trial registration Registry number Netherlands Trial Register (www.trialregister.nl): NTR5060. Registered 13 February 2015.

Background: In children with Brachial Plexus Birth Injury, a non-use of the affected upper limb despite sufficient capacity, is sometimes observed, called developmental disregard. The combination of modified constraint-induced-movement-therapy with bimanual training is frequently applied to overcome developmental disregard in unilateral Cerebral Palsy. In the current study the effects of the combination of modified constraint-induced-movement-therapy with bimanual training are investigated in children with Brachial Plexus Birth Injury in comparison to children with unilateral Cerebral Palsy. We hypothesize that the combination of modified constraint-induced-movement-therapy with bimanual training is effective in Brachial Plexus Birth Injury. Methods: Data of 19 children with Brachial Plexus Birth Injury (Mage: 4.1 years) and 18 with unilateral Cerebral Palsy (Mage: 4.5 years) were compared. The effects of modified constraint-induced-movement-therapy with bimanual training (54 h modified constraint-induced-movement-therapy, 18 h bimanual training, 8–10 weeks) was investigated by assessing spontaneous affected-upper-limb-use (“Assisting Hand Assessment”), manual abilities (“ABILHAND-kids”) and subjective performance and satisfaction of problematic bimanual activities (“Canadian Occupational Performance Measure”) at three time points (pre-treatment, post-treatment, follow-up). This data was analyzed using repeated-measures analysis. Results: Children with Brachial Plexus Birth Injury showed significant improvements on all outcome measures following modified constraint-induced-movement-therapy with bimanual training. These results were comparable to those observed in the group of children with unilateral Cerebral Palsy. Discussion: These results suggest that modified constraint-induced-movement-therapy with bimanual training is effective in Brachial Plexus Birth Injury. They indicate a comparable improved bimanual performance in children with Brachial Plexus Birth Injury than in unilateral Cerebral Palsy and suggests that both groups of children have affectively overcome their developmental disregard. IMPLICATIONS FOR REHABILITATION • Children with Brachial Plexus Birth Injury frequently experience difficulties in activities of daily living. • It has recently been suggested that children with Brachial Plexus Birth Injury may also show a non-use of the affected upper limb despite sufficient capacity, called developmental disregard. • Children with Brachial Plexus Birth Injury and developmental disregard might therefore benefit from intensive therapies aimed at overcoming developmental disregard, originally developed for children with unilateral Cerebral Palsy. • A combination of modified Constrained-Induced Movement Therapy with intensive Bimanual Training has shown to be affective in children with unilateral Cerebral Palsy. • In a small sample, this study shows that a combination of modified Constrained-Induced Movement Therapy with intensive bimanual training is effective in children with Brachial Plexus Birth Injury, comparable and even more than in unilateral Cerebral Palsy.

Contains fulltext : 218678.pdf (Publisher’s version ) (Closed access) BACKGROUND: Stroke survivors often show reduced walking velocity and gait asymmetry. These gait abnormalities are associated with reduced propulsion of the paretic leg. This review aimed to provide an overview of the potential effectiveness of post-stroke rehabilitation interventions to improve paretic propulsion, ankle kinetics and walking velocity. METHODS: A systematic search was performed in Pubmed, Web of Science, Embase, and Pedro. Studies were eligible if they reported changes in propulsion measures (impulse, peak value and symmetry ratios) or ankle kinetics (moment and power) following intervention in stroke survivors (group size >/=10). Study selection, data extraction and quality assessment were performed independently by two authors. FINDINGS: A total of 28 studies were included, of which 25 studies applied exercise interventions, two studies focused on surgical interventions, and one on non-invasive brain stimulation. The number of high-quality trials was limited (N=6; score Downs and Black scale >/=19). Propulsion measures were the primary outcome in eight studies. In general, mixed results were reported with 14 interventions yielding improvements in propulsion and ankle kinetics. In contrast, gains in walking velocity were observed in the vast majority of studies (N=20 out of 23). INTERPRETATION: Interventions that yielded gains in propulsion appeared to have in common that they challenged and/or enabled the utilization of latent propulsive capacity of the paretic leg during walking. Walking speed generally increased, regardless of the observed change in propulsion, suggesting the use of compensatory mechanisms. Findings should, however, be interpreted with some caution, as the evidence base for this emerging focus of rehabilitation is limited.

Background Dysphagia is reported in patients with Duchenne or Becker muscular dystrophy. Our clinical experience suggests that, compared with Duchenne patients, impaired mastication and swallowing occur early in Becker patients relative to their skeletal muscle involvement. The aim of this study was to assess dysphagia in Duchenne and Becker patients in relation to ambulatory capacity. Methods In patients in the early ambulatory stage, clinical symptoms, quantitative muscle ultrasound of the orofacial muscles, and maximum bite force were assessed. The 6‐Minute Walk Test (6MWT) was used to measure ambulatory capacity. Results Eleven Duchenne and 11 Becker patients were included. Although Becker patients had a greater 6MWT distance than Duchenne patients, the occurrence of mastication and swallowing difficulties was similar. The temporalis muscle was significantly thicker in Becker patients. Conclusions Clinicians should be aware of dysphagia in both groups, even when ambulation is still well preserved.

Background Transcranial direct current stimulation (tDCS) is a non-invasive brain stimulation technique that has shown promise for rehabilitation after stroke. Ipsilesional anodal tDCS (a-tDCS) over the motor cortex increases corticospinal excitability, while contralesional cathodal tDCS (c-tDCS) restores interhemispheric balance, both resulting in offline improved reaction times of delayed voluntary upper-extremity movements. We aimed to investigate whether tDCS would also have a beneficial effect on delayed leg motor responses after stroke. In addition, we identified whether variability in tDCS effects was associated with the level of leg motor function. Methods In a cross-over design, 13 people with chronic stroke completed three 15-min sessions of anodal, cathodal and sham stimulation over the primary motor cortex on separate days in an order balanced across participants. Directly after stimulation, participants performed a comprehensive set of lower-extremity tasks involving the paretic tibialis anterior (TA): voluntary ankle-dorsiflexion, gait initiation, and backward balance perturbation. For all tasks, TA onset latencies were determined. In addition, leg motor function was determined by the Fugl-Meyer Assessment – leg score (FMA-L). Repeated measures ANOVA was used to reveal tDCS effects on reaction times. Pearson correlation coefficients were used to establish the relation between tDCS effects and leg motor function. Results For all tasks, TA reaction times did not differ across tDCS sessions. For gait initiation and backward balance perturbation, differences between sham and active stimulation (a-tDCS or c-tDCS) did not correlate with leg motor function. Yet, for ankle dorsiflexion, individual reaction time differences between c-tDCS and sham were strongly associated with FMA-L, with more severely impaired patients exhibiting slower paretic reaction times following c-tDCS. Conclusion We found no evidence for offline tDCS-induced benefits. Interestingly, we found that c-tDCS may have unfavorable effects on voluntary control of the paretic leg in severely impaired patients with chronic stroke. This finding points at potential vicarious control from the unaffected hemisphere to the paretic leg. The absence of tDCS-induced effects on gait and balance, two functionally relevant tasks, shows that such motor behavior is inadequately stimulated by currently used tDCS applications. Trial registration The study is registered in the Netherlands Trial Register (NL5684; April 13th, 2016).

Objective To identify the role of hyperexcitable short-latency stretch reflexes (SLRs) on balance control in people with hereditary spastic paraplegia (PwHSP). Methods Sixteen PwHSP with triceps surae spasticity and 9 healthy control subjects were subjected to toes-up support-surface perturbations. EMG data were recorded from gastrocnemius, soleus and tibialis anterior. Furthermore, center-of-mass trajectories were recorded. Results PwHSP were less able to withstand the perturbations. Triceps surae SLRs (40–80 ms post perturbation) in PwHSP were increased compared to healthy subjects. Furthermore, a sustained triceps surae EMG activity at 220–320 ms post perturbation was observed in PwHSP, whereas control subjects demonstrated suppression of triceps surae activity. Center of mass trajectories started to diverge between PwHSP and controls only after ∼500 ms, with greater excursions being observed in the PwHSP. Conclusions The present results confirm that balance control is impaired in PwHSP. However, the late instant of center of mass divergence argues against a direct, causative role of hyperexcitable SLRs in the triceps surae. Significance We postulate that enhanced short-latency stretch reflexes of the triceps surae do not underlie poor balance control in PwHSP. Instead, we suggest the lack of suppression of later triceps surae activity to be the main cause.

Objective: To investigate the concomitant effects of two patient-directed interventions for post-stroke depressive symptoms on caregivers’ well-being. Design: Secondary analyses of the results of a randomized controlled trial. Subjects: Fifty caregivers of stroke patients receiving outpatient rehabilitation. Interventions: Stroke patients and their caregivers were randomly allocated to either cognitive-behavioural therapy augmented with movement or occupational therapy ( n = 23) or computerized cognitive training ( n = 27) to alleviate depressive symptoms in patients. Main measures: Emotional burden (Involvement Evaluation Questionnaire), practical burden (Caregiver Strain Index), mental health (General Health Questionnaire) and emotional complaints (Hospital Anxiety and Depression Scale). Results: Caregivers of patients who received cognitive-behavioural therapy reported significantly higher mental health levels (mean difference (MD) = 1.78, 95% confidence interval (CI) = 0.43–3.13, P = 0.01) and less worrying about patients’ well-being (MD = 1.9, 95% CI = 0.56–3.24, P Conclusion: The results suggest that augmented cognitive-behavioural therapy aimed at improving patients’ emotional, behavioural and social functioning positively affects some aspects of caregivers’ well-being.

Background Implanted peroneal functional electrical stimulation (FES) is an effective alternative treatment to ankle-foot orthosis (AFO) in people with drop foot after stroke. With FES no constraints on ankle mobility are imposed which might particularly be exploited in challenging walking environments that require adaptations of the gait pattern to environmental disturbances. Research question Is gait adaptability, by means of the capacity to avoid sudden obstacles while walking on a treadmill, superior with implanted FES compared to AFO in people with drop foot after stroke? Methods A 4-channel peroneal nerve stimulator (ActiGait®) was implanted in 22 persons with stroke (>6 months) who regularly used an AFO. Gait adaptability was tested with an obstacle avoidance task on an instrumented treadmill up to 26 weeks (n = 10) or 52 weeks (n = 12) after FES-system activation. At assessments, 30 trials, in which obstacles were suddenly dropped onto the treadmill in front of the paretic leg, were recorded with each device (FES / AFO). Trials were grouped by available response times (ART) and success rates were calculated. The effect of device, ART and follow up time on success rates was tested using generalized estimated equations. Nonparametric correlations were calculated to associate changes in success rates with clinimetrics. Results Success rates of obstacle avoidance were higher when participants used their FES system compared to AFO (Δ4.7%, p = 0.03), which effect was largest for longest ARTs (Δ15%, p = 0.03). Participants with greater motor impairment of the paretic leg showed greater benefit from FES (rs=-0.49, p = 0.04). Significance FES has been found equally effective as AFO in improving walking speed of people with drop foot after stroke. We now present superior walking performance in a complex walking environment for implanted peroneal FES compared to AFO. These findings underline the importance of using gait assessments that require interplay with the environment, besides assessment of stationary walking, in community ambulators.

Gait impairments in people with Charcot Marie Tooths disease are the combined result of ankle-foot deformities, muscle weakness, and somatosensory impairments. People with Charcot-Marie-Tooth disease often experience pain and difficulties when walking, especially barefoot. They also trip and fall frequently and have a lower than normal gait speed and distance. Because these gait impairments and related complaints are disabling, clinical management aimed at improving gait is important. Management involves both conservative and surgical treatment options, each with limited scientific evidence. However, a treatment algorithm that describes both conservative and surgical treatment options is currently lacking. This study sets out a step-wise treatment algorithm, based on evidence, if available, and otherwise reflecting practice-based experience. The treatment algorithm will be of value in daily clinical practice, and will serve as a template for future research.

Background. Many stroke survivors suffer from leg muscle paresis, resulting in asymmetrical gait patterns, negatively affecting balance control and energy cost. Interventions targeting asymmetry early after stroke may enhance recovery of walking. Objective. To determine the feasibility and preliminary efficacy of up to 10 weeks of gait training assisted by multichannel functional electrical stimulation (MFES gait training) applied to the peroneal nerve and knee flexor or extensor muscle on the recovery of gait symmetry and walking capacity in patients starting in the subacute phase after stroke. Methods. Forty inpatient participants (≤31 days after stroke) were randomized to MFES gait training (experimental group) or conventional gait training (control group). Gait training was delivered in 30-minute sessions each workday. Feasibility was determined by adherence (≥75% sessions) and satisfaction with gait training (score ≥7 out of 10). Primary outcome for efficacy was step length symmetry. Secondary outcomes included other spatiotemporal gait parameters and walking capacity (Functional Gait Assessment and 10-Meter Walk Test). Linear mixed models estimated treatment effect postintervention and at 3-month follow-up. Results. Thirty-seven participants completed the study protocol (19 experimental group participants). Feasibility was confirmed by good adherence (90% of the participants) and participant satisfaction (median score 8). Both groups improved on all outcomes over time. No significant group differences in recovery were found for any outcome. Conclusions. MFES gait training is feasible early after stroke, but MFES efficacy for improving step length symmetry, other spatiotemporal gait parameters, or walking capacity could not be demonstrated. Trial Registration. Netherlands Trial Register (NTR4762).

Contains fulltext : 238546.pdf (Publisher’s version ) (Open Access)

Background After stroke, some individuals have latent, propulsive capacity of the paretic leg, that can be elicited during task-specific gait training. The aim of this proof-of-concept study was to investigate the effect of five-week robotic gait training for improving propulsion symmetry by increasing paretic propulsion in chronic stroke survivors. Methods Twenty-nine individuals with chronic stroke and impaired paretic propulsion (≥ 8% difference in paretic vs. non-paretic propulsive impulse) were enrolled. Participants received ten 60-min sessions of individual robotic gait training targeting paretic propulsion (five weeks, twice a week), complemented with home exercises (15 min/day) focusing on increasing strength and practicing learned strategies in daily life. Propulsion measures, gait kinematics and kinetics, self-selected gait speed, performance of functional gait tasks, and daily-life mobility and physical activity were assessed five weeks (T0) and one week (T1) before the start of intervention, and one week (T2) and five weeks (T3) after the intervention period. Results Between T0 and T1, no significant differences in outcomes were observed, except for a marginal increase in gait speed (+ 2.9%). Following the intervention, propulsion symmetry (+ 7.9%) and paretic propulsive impulse had significantly improved (+ 8.1%), whereas non-paretic propulsive impulse remained unchanged. Larger gains in propulsion symmetry were associated with more asymmetrical propulsion at T0. In addition, following the intervention significantly greater paretic trailing limb angles (+ 6.6%) and ankle plantarflexion moments (+ 7.1%) were observed. Furthermore, gait speed (+ 7.2%), 6-Minute Walk Test (+ 6.4%), Functional Gait Assessment (+ 6.5%), and daily-life walking intensity (+ 6.9%) had increased following the intervention. At five-week follow-up (T3), gains in all outcomes were retained, and gait speed had further increased (+ 3.6%). Conclusions The post-intervention gain in paretic propulsion did not only translate into improved propulsion symmetry and gait speed, but also pertained to performance of functional gait tasks and daily-life walking activity levels. These findings suggest that well-selected chronic stroke survivors may benefit from task-specific targeted training to utilize the residual propulsive capacity of the paretic leg. Future research is recommended to establish simple baseline measures for identification of individuals who may benefit from such training and confirm benefits of the used training concepts in a randomized controlled trial. Trial registration: Registry number ClinicalTrials.gov (www.clinicaltrials.gov): NCT04650802, retrospectively registered 3 December 2020.

Background: Exoskeleton use by people with complete spinal cord injury (SCI) in daily life is still a challenge. To optimize its daily life use, a better understanding of the purpose of use and the accompanying improvements are needed. The perspective of experienced exoskeleton users provides an important contribution to the improvement of future exoskeletons.Methods: Face-to-face semi-structured interviews were held with 13 people with SCI, who were experienced exoskeleton users. Interviews were audio-taped, transcribed and analysed thematically.Results: Participants expressed three future purposes of exoskeleton use: for daily activities, exercise, and social interaction. Exoskeleton use during daily activities was the ultimate goal. Therefore, the future exoskeleton should be: easy to use, small and lightweight, tailor made, safe, comfortable, less distinctive, durable, and affordable. Improving the ease of use was relevant for all purposes, for all participants. The other suggestions for improvement varied depending on the purpose of use and the participant. Conclusions: Increasingly more advanced improvements to future exoskeletons are needed to transition from an exercise purpose, to social interaction, and ultimately use during daily activities. In the current study, detailed suggestions for improvements from experienced exoskeleton users have been made. Only when multiple of these suggestions are adjusted, can the exoskeleton be used to its full potential.

Contains fulltext : 225521.pdf (Publisher’s version ) (Open Access) A consequence of a complete spinal cord injury (SCI) is the loss of gait capacity. Wearable exoskeletons for the lower extremity enable household and community ambulation in people with SCI. This study assessed the amount, purpose, and location of exoskeleton use in the home and community environment, without any restrictions. The number of steps taken was read from the exoskeleton software. Participants kept a daily logbook, and completed two user experience questionnaires (Quebec User Evaluation of Satisfaction with assistive Technology (D-QUEST) and System Usability Scale (SUS)). Fourteen people with a complete SCI used the ReWalk exoskeleton a median of 9 (range [1-15]) out of 16 ([12-21]) days, in which participants took a median of 3,226 ([330-28,882]) steps. The exoskeleton was mostly used for exercise purposes (74%) and social interaction (20%). The main location of use was outdoors (48%). Overall, participants were satisfied with the exoskeleton (D-QUEST 3.7 ± 0.4) and its usability (SUS 72.5 [52.5-95.0]). Participants with complete SCI report satisfaction with the exoskeleton for exercise and social interaction in the home and community, but report limitations as an assistive device during daily life.

Contains fulltext : 229732.pdf (Publisher’s version ) (Closed access)

Background Although it is well-established that osteoarthritis (OA) impairs daily-life gait, objective gait assessments are not part of routine clinical evaluation. Wearable inertial sensors provide an easily accessible and fast way to routinely evaluate gait quality in clinical settings. However, during these assessments, more complex and meaningful aspects of daily-life gait, including turning, dual-task performance, and upper body motion, are often overlooked. The aim of this study was therefore to investigate turning, dual-task performance, and upper body motion in individuals with knee or hip OA in addition to more commonly assessed spatiotemporal gait parameters using wearable sensors. Methods Gait was compared between individuals with unilateral knee (n = 25) or hip OA (n = 26) scheduled for joint replacement, and healthy controls (n = 27). For 2 min, participants walked back and forth along a 6-m trajectory making 180° turns, with and without a secondary cognitive task. Gait parameters were collected using 4 inertial measurement units on the feet and trunk. To test if dual-task gait, turning, and upper body motion had added value above spatiotemporal parameters, a factor analysis was conducted. Effect sizes were computed as standardized mean difference between OA groups and healthy controls to identify parameters from these gait domains that were sensitive to knee or hip OA. Results Four independent domains of gait were obtained: speed-spatial, speed-temporal, dual-task cost, and upper body motion. Turning parameters constituted a gait domain together with cadence. From the domains that were obtained, stride length (speed-spatial) and cadence (speed-temporal) had the strongest effect sizes for both knee and hip OA. Upper body motion (lumbar sagittal range of motion), showed a strong effect size when comparing hip OA with healthy controls. Parameters reflecting dual-task cost were not sensitive to knee or hip OA. Conclusions Besides more commonly reported spatiotemporal parameters, only upper body motion provided non-redundant and sensitive parameters representing gait adaptations in individuals with hip OA. Turning parameters were sensitive to knee and hip OA, but were not independent from speed-related gait parameters. Dual-task parameters had limited additional value for evaluating gait in knee and hip OA, although dual-task cost constituted a separate gait domain. Future steps should include testing responsiveness of these gait domains to interventions aiming to improve mobility.

Contains fulltext : 229506.pdf (Publisher’s version ) (Open Access) Purpose: Little is known concerning the impact of chronic spasticity on physical activities, social participation, and well-being, and whether patients' needs are addressed by current treatments. This study aims to investigate these lacunas in persons with a pure form of hereditary spastic paraplegia (HSP), in whom spasticity is a prominent symptom.Methods: Fourteen patients with a pure form of HSP were interviewed. These interviews were recorded, verbally transcribed, and thematically analyzed.Results: Four themes were identified which can be reflected by the phrases: (1) 'I stumble', (2) 'I struggle', (3) 'I feel ashamed', and (4) 'I need support'. Balance and gait problems led to limitations in domestic activities, employment, and recreation. 'Stumbling' also occurred due to pain, stiffness, and fatigue. Struggling was related to the continuous need for adaptation strategies, including the abandonment of some activities. Participants further reported feelings of shame, fear, and frustration. Lastly, they needed more support in daily activities than currently provided.Conclusion: Besides treating spasticity-related motor impairments, patients with HSP need practical support for optimizing their physical activities and social participation. They also seek attention for the non-motor consequences of their chronic spasticity to improve their well-being. Patient-reported outcomes might help to address these needs.Implications for rehabilitationAccording to patients with hereditary spastic paraplegia, interventions for spasticity should not only be aimed at reducing motor impairments, but also on reducing pain and fatigue, improving nighttime rest and general well-being, and optimizing the performance of relevant personal activities.Medical, role and emotional management in patients with hereditary spastic paraplegia can be improved only when individual needs are identified and monitored over the course of the disease.Besides assessment of bodily functions and physical capacities, systematic evaluation of patient-reported outcomes will help both patients and professionals to monitor the functional impact of disease progression and to evaluate the effects of interventions aimed at retarding this progression.

Background Hereditary spastic paraplegia (HSP) is a group of inherited disorders characterized by progressive spastic paresis of the lower limbs. Treatment is often focused on reducing spasticity and its physical consequences. To better address individual patients’ needs, we investigated a broad range of experienced complaints, activity limitations, and loss of motor capacities in pure HSP. In addition, we aimed to identify patient characteristics that are associated with increased fall risk and/or reduced walking capacity. Methods We developed and distributed an HSP-specific online questionnaire in the Netherlands. A total of 109 out of 166 questionnaires returned by participants with pure HSP were analyzed. Results Participants experienced the greatest burden from muscle stiffness and limited standing and walking activities, while 72% reported leg and/or back pain. Thirty-five and 46% reported to use walking aids (e.g. crutches) indoors and outdoors, respectively; 57% reported a fall incidence of at least twice a year (‘fallers’); in 51% a fall had led to an injury at least once; and 73% reported fear of falling. Duration of spasticity and incapacity to rise from the floor were positively associated with being a ‘faller’, whereas non-neurological comorbidity and wheelchair use were negatively associated. Higher age, experienced gait problems, not being able to stand for 10 min, and incapacity to open a heavy door showed a negative association with being a ‘walker without aids’ (> 500 m). Conclusions Our results emphasize the large impact of spastic paraparesis on the lives of people with pure HSP and contribute to a better understanding of possible targets for rehabilitation.

Background Falls are one of the most common complications after stroke, with a reported incidence ranging between 7% in the first week and 73% in the first year post stroke. This is an updated version of the original Cochrane Review published in 2013. Objectives To evaluate the effectiveness of interventions aimed at preventing falls in people after stroke. Our primary objective was to determine the effect of interventions on the rate of falls (number of falls per person-year) and the number of fallers. Our secondary objectives were to determine the effects of interventions aimed at preventing falls on 1) the number of fall-related fractures; 2) the number of fall-related hospital admissions; 3) near-fall events; 4) economic evaluation; 5) quality of life; and 6) adverse effects of the interventions. Search methods We searched the trials registers of the Cochrane Stroke Group (September 2018) and the Cochrane Bone, Joint and Muscle Trauma Group (October 2018); the Cochrane Central Register of Controlled Trials (CENTRAL; 2018, Issue 9) in the Cochrane Library; MEDLINE (1950 to September 2018); Embase (1980 to September 2018); CINAHL (1982 to September 2018); PsycINFO (1806 to August 2018); AMED (1985 to December 2017); and PEDro (September 2018). We also searched trials registers and checked reference lists. Selection criteria Randomised controlled trials of interventions where the primary or secondary aim was to prevent falls in people after stroke. Data collection and analysis Two review authors (SD and WS) independently selected studies for inclusion, assessed trial quality and risk of bias, and extracted data. We resolved disagreements through discussion, and contacted study authors for additional information where required. We used a rate ratio and 95% confidence interval (CI) to compare the rate of falls (e.g. falls per person-year) between intervention and control groups. For risk of falling we used a risk ratio and 95% CI based on the number of people falling (fallers) in each group. We pooled results where appropriate and applied GRADE to assess the quality of the evidence. Main results We included 14 studies (of which six have been published since the first version of this review in 2013), with a total of 1358 participants. We found studies that investigated exercises, predischarge home visits for hospitalised patients, the provision of single lens distance vision glasses instead of multifocal glasses, a servo-assistive rollator and non-invasive brain stimulation for preventing falls. Exercise compared to control for preventing falls in people after stroke The pooled result of eight studies showed that exercise may reduce the rate of falls but we are uncertain about this result (rate ratio 0.72, 95% CI 0.54 to 0.94, 765 participants, low-quality evidence). Sensitivity analysis for single exercise interventions, omitting studies using multiple/multifactorial interventions, also found that exercise may reduce the rate of falls (rate ratio 0.66, 95% CI 0.50 to 0.87, 626 participants). Sensitivity analysis for the effect in the chronic phase post stroke resulted in little or no difference in rate of falls (rate ratio 0.58, 95% CI 0.31 to 1.12, 205 participants). A sensitivity analysis including only studies with low risk of bias found little or no difference in rate of falls (rate ratio 0.88, 95% CI 0.65 to 1.20, 462 participants). Methodological limitations mean that we have very low confidence in the results of these sensitivity analyses. For the outcome of number of fallers, we are very uncertain of the effect of exercises compared to the control condition, based on the pooled result of 10 studies (risk ratio 1.03, 95% CI 0.90 to 1.19, 969 participants, very low quality evidence). The same sensitivity analyses as described above gives us very low certainty that there are little or no differences in number of fallers (single interventions: risk ratio 1.09, 95% CI 0.93 to 1.28, 796 participants; chronic phase post stroke: risk ratio 0.94, 95% CI 0.73 to 1.22, 375 participants; low risk of bias studies: risk ratio 0.96, 95% CI 0.77 to 1.21, 462 participants). Other interventions for preventing falls in people after stroke We are very uncertain whether interventions other than exercise reduce the rate of falls or number of fallers. We identified very low certainty evidence when investigating the effect of predischarge home visits (rate ratio 0.85, 95% CI 0.43 to 1.69; risk ratio 1.48, 95% CI 0.71 to 3.09; 85 participants), provision of single lens distance glasses to regular wearers of multifocal glasses (rate ratio 1.08, 95% CI 0.52 to 2.25; risk ratio 0.74, 95% CI 0.47 to 1.18; 46 participants) and a servo-assistive rollator (rate ratio 0.44, 95% CI 0.16 to 1.21; risk ratio 0.44, 95% CI 0.16 to 1.22; 42 participants). Finally, transcranial direct current stimulation (tDCS) was used in one study to examine the effect on falls post stroke. We have low certainty that active tDCS may reduce the number of fallers compared to sham tDCS (risk ratio 0.30, 95% CI 0.14 to 0.63; 60 participants). Authors' conclusions At present there exists very little evidence about interventions other than exercises to reduce falling post stroke. Low to very low quality evidence exists that this population benefits from exercises to prevent falls, but not to reduce number of fallers. Fall research does not in general or consistently follow methodological gold standards, especially with regard to fall definition and time post stroke. More well-reported, adequately-powered research should further establish the value of exercises in reducing falling, in particular per phase, post stroke. ispartof: Cochrane Database Of Systematic Reviews vol:10 issue:10 ispartof: location:England status: published

Objective: Post-stroke depressive symptoms have a vast individual and societal impact. However, research into interventions for such symptoms show contradictory results; it is unclear what works for which patients. In addition, clinical prediction tools are lacking. This study aimed to develop a prognostic index model for treatment outcome in patients with post-stroke depressive symptoms.Methods: Data from a randomized controlled trial (n = 61) evaluating 2 interventions for post-stroke depressive symptoms were used to predict post-treatment post-stroke depressive symptoms and participation. From 18 pre-treatment variables of patients and caregivers, predictors were selected using elastic net regression. Based on this selection, prognostic index scores (i.e. predictions) for both out comes were computed for each individual patient.Results: The depression model included all pre-treatment variables, explaining 44% of the variance. The strongest predictors were: lesion location, employment, participation, comorbidities, mobility, sex, and pre-treatment depression. Six predictors of post-treatment participation were identified, explaining 51% of the variance: mobility, pre-treatment participation, age, satisfaction with participation, caregiver strain, and psychological distress of the spouse. The cross-validated prognostic index scores correlated highly with the actual outcome scores (depression: correlation = 0.672; participation: correlation = 0.718).Conclusion: Post-stroke depressive symptoms form a complex and multifactorial problem. Treatment outcome is influenced by the characteristics of the stroke, the patients, and their spouses. The results show that psychological distress is probably no obstacle to attempting to improve participation. The personalized predictions (prognostic index scores) of treatment outcome show promising results, which, after further replication and validation, could aid clinicians with treatment selection.

Corticospinal lesions cause impairments in voluntary motor control. Recent findings suggest that some degree of voluntary control may be taken over by a compensatory pathway involving the reticulospinal tract. In humans, evidence for this notion mainly comes from StartReact studies. StartReact is the acceleration of reaction times by a startling acoustic stimulus (SAS) simultaneously presented with the imperative stimulus. As previous StartReact studies mainly focused on isolated single-joint movements, the question remains whether the reticulospinal tract can also be utilized for controlling whole-body movements. To investigate reticulospinal control, we applied the StartReact paradigm during gait initiation in 12 healthy controls and 12 patients with ‘pure’ hereditary spastic paraplegia (HSP; i.e., retrograde axonal degeneration of corticospinal tract). Participants performed three consecutive steps in response to an imperative visual stimulus. In 25% of 16 trials a SAS was applied. We determined reaction times of muscle (de)activation, anticipatory postural adjustments (APA) and steps. Without SAS, we observed an overall delay in HSP patients compared to controls. Administration of the SAS accelerated tibialis anterior and rectus femoris onsets in both groups, but more so in HSP patients, resulting in (near-)normal latencies. Soleus offsets were accelerated in controls, but not in HSP patients. The SAS also accelerated APA and step reaction times in both groups, yet these did not normalize in the HSP patients. The reticulospinal tract is able to play a compensatory role in voluntary control of whole-body movements, but seems to lack the capacity to inhibit task-inappropriate muscle activity in patients with corticospinal lesions.

Item does not contain fulltext BACKGROUND: Trunk control is essential during seated activities. The trunk interacts with the upper extremities (UE) and head by being part of a kinematic chain and by providing a stable basis. When trunk control becomes impaired, it may have consequences for the execution of UE tasks. AIM: To review trunk involvement in body movement and stability when performing seated activities and its relation with UE and head movements in neurological patients with a flaccid trunk, with a focus on childhood and development with age. METHODS AND PROCEDURES: A search using PubMed was conducted and 32 out of 188 potentially eligible articles were included. OUTCOMES AND RESULTS: Patients with a flaccid trunk (e.g. with spinal cord injury or cerebral palsy) tend to involve the trunk earlier while reaching than healthy persons. Different balance strategies are observed in different types of patients, like using the contralateral arm as counterweight, eliminating degrees of freedom, or reducing movement speed. CONCLUSIONS AND IMPLICATIONS: The key role of the trunk in performing activities should be kept in mind when developing interventions to improve seated task performance in neurological patients with a flaccid trunk.

Defective muscle coordination for balance recovery may contribute to stroke survivors’ propensity for falling. Thus, we investigated deficits in muscle coordination for postural control and their association to body sway following balance perturbations in people with stroke. Specifically, we compared the automatic postural responses of 8 leg and trunk muscles recorded bilaterally in unimpaired individuals and those with mild to moderate impairments after unilateral supratentorial lesions (>6 months). These responses were elicited by unexpected floor translations in 12 directions. We extracted motor modules (ie, muscle synergies) for each leg using nonnegative matrix factorization. We also determined the magnitude of perturbation-induced body sway using a single-link inverted pendulum model. Whereas the number of motor modules for balance was not affected by stroke, those formed by muscles with long latency responses were replaced by atypically structured paretic motor modules (atypical muscle groupings), which hints at direct cerebral involvement in long-latency feedback responses. Other paretic motor modules had intact structure but were poorly recruited, which is indicative of indirect cerebral control of balance. Importantly, these paretic deficits were strongly associated with postural instability in the preferred activation direction of the impaired motor modules. Finally, these deficiencies were heterogeneously distributed across stroke survivors with lesions in distinct locations, suggesting that different cerebral substrates may contribute to balance control. In conclusion, muscle coordination deficits in the paretic limb of stroke survivors result in direction-specific postural instability, which highlights the importance of targeted interventions to address patient-specific balance impairments.

Item does not contain fulltext The aim of this research was to study impairments, activity limitations and participation restrictions due to upper limb involvement in people with four different types of neuromuscular disorders (NMD) - FacioScapuloHumeral Dystrophy (FSHD), Limb-Girdle Muscular Dystrophy (LGMD), Spinal Muscular Atrophy (SMA) and Duchenne Muscular Dystrophy (DMD) - and to investigate whether common or different profiles could be identified. Total of 267 respondents with NMD from the Netherlands answered a set of questionnaires covering upper limb impairments (pain and stiffness), activity limitations and participation restrictions. Pain was most often reported by persons with FSHD. Problems in the FSHD group were mainly characterized by relatively high scores for pain and stiffness and low scores for activity limitations. People with LGMD reported also low scores for activity limitations. Conversely, people with SMA and DMD experienced in general relatively low scores for pain and stiffness and high scores for activity limitations. Although people with FSHD and LGMD had relatively few activity limitations, all NMD groups experienced restrictions when participating in social activities. Our results indicate specific profiles of upper limb function in different types of NMD. While the profile observed in persons with FSHD seems to reflect overuse, the profile seen in persons with DMD and SMA is suspicious of disuse, each requiring a specific rehabilitation strategy.

Duchenne muscular dystrophy can lead to upper extremity limitations, pain and stiffness. In a previous study, these domains have been investigated using extensive questionnaires, which are too time-consuming for clinical practice. This study aimed at gaining insight into the underlying dimensions of these questionnaires, and to construct a short questionnaire that can be used for clinical assessment.Exploratory factor analysis was performed on the responses of 213 participants to a web-based survey to find the underlying dimensions in the Capabilities of Upper Extremity questionnaire, the ABILHAND questionnaire, and questionnaires regarding pain and stiffness. Based on these underlying dimensions, a stepwise approach was formulated. In addition, construct validity of the factors was investigated.In total, 14 factors were identified. All had high internal consistency (Cronbach's alpha0.89) and explained 80-88% of the variance of the original questionnaires. Construct validity was supported, because participants in the early ambulatory stage performed significantly better (p 0.001) than participants in the late non-ambulatory stage.The factors identified from the set of questionnaires provide a valid representation of upper extremity function, pain and stiffness in Duchenne muscular dystrophy. Based on the factor commonalities, the Upper Limb Short Questionnaire was formulated. Implications for Rehabilitation New insights into the underlying dimensions of upper extremity function, pain and stiffness in Duchenne muscular dystrophy are gained. Fourteen factors, with good internal consistency and construct validity, are identified regarding upper extremity function, pain and stiffness in Duchenne muscular dystrophy. Based on these factors, the Upper Limb Short Questionnaire is presented. The Upper Limb Short Questionnaire can be used as an identifier of arm-hand limitations and the start of more thorough clinical investigation.

Contains fulltext : 176935.pdf (Publisher’s version ) (Open Access) BACKGROUND AND OBJECTIVE: Postural muscle responses are often impaired after stroke. We aimed to identify the contribution of deficits in very early postural responses to poorer reactive balance capacity, with a particular focus on reactive stepping as a key strategy for avoiding falls. METHODS: A total of 34 chronic stroke survivors and 17 controls were subjected to translational balance perturbations in 4 directions. We identified the highest perturbation intensity that could be recovered without stepping (single stepping threshold [SST]) and with maximally 1 step (multiple stepping threshold [MST]). We determined onset latencies and response amplitudes of 7 leg muscles bilaterally and identified associations with balance capacity. RESULTS: People with stroke had a lower MST than controls in all directions. Side steps resulted in a higher lateral MST than crossover steps but were less common toward the paretic side. Postural responses were delayed and smaller in amplitude on the paretic side only. We observed the strongest associations between gluteus medius (GLUT) onset and amplitude and MST toward the paretic side ( R2 = 0.33). Electromyographic variables were rather weakly associated with forward and backward MSTs ( R2 = 0.10-0.22) and with SSTs ( R2 = 0.08-0.15). CONCLUSIONS: Delayed and reduced paretic postural responses are associated with impaired reactive stepping after stroke. Particularly, fast and vigorous activity of the GLUT is imperative for overcoming large sideways perturbations, presumably because it facilitates the effective use of side steps. Because people with stroke often fall toward the paretic side, this finding indicates an important target for training.

Contains fulltext : 174824.pdf (Publisher’s version ) (Open Access) INTRODUCTION: Balance impairments are common in patients with hereditary spastic paraplegia and are among the most debilitating symptoms, as they frequently result in falls and fall-related injuries. Several features of hereditary spastic paraplegia contribute to balance impairments and multiple treatment options exist. However, an overview of these underlying mechanisms and their treatment is currently lacking. METHODS: This paper reviews the pathophysiology, diagnostic workup, and management of balance impairments in hereditary spastic paraplegia. Recommendations are based on scientific evidence, when available, and otherwise reflect practice-based evidence supported by clinical experience. CONCLUSION: Through diligent history-taking and clinical examination, followed by multidisciplinary treatment tailored to the identified underlying mechanisms, balance capacities can be improved in patients with hereditary spastic paraplegia and at least a proportion of falls can be prevented.

Background Neuralgic amyotrophy (NA) is a distinct peripheral neurological disorder of the brachial plexus with a yearly incidence of 1/1000, which is characterised by acute severe upper extremity pain. Weakness of the stabilising shoulder muscles in the acute phase leads to compensatory strategies and abnormal motor control of the shoulder - scapular dyskinesia. Despite peripheral nerve recovery, scapular dyskinesia often persists, leading to debilitating residual complaints including pain and fatigue. Evidence suggests that persistent scapular dyskinesia in NA may result from maladaptive cerebral neuroplasticity, altering motor planning. Currently there is no proven effective causative treatment for the residual symptoms in NA. Moreover, the role of cerebral mechanisms in persistent scapular dyskinesia remains unclear. Methods NA-CONTROL is a single-centre, randomised controlled trial comparing specific rehabilitation to usual care in NA. The rehabilitation programme combines relearning of motor control, targeting cerebral mechanisms, with self-management strategies. Fifty patients will be included. Patients are recruited through the Radboud university medical center Nijmegen, the Netherlands. Patients with a (suspected) diagnosis of NA, with lateralized symptoms and scapular dyskinesia in the right upper extremity, who are 18 years or older and not in the acute phase can be included. The primary outcome is the Shoulder Rating Questionnaire score, which measures functional capability of the upper extremity. Secondary clinical outcomes include measures of pain, fatigue, participation, reachable workspace, muscle strength and quality of life. In addition, motor planning is assessed with first-person motor imagery and functional magnetic resonance imaging. In a sub-study the patients are compared to 25 healthy participants, to determine the involvement of cerebral mechanisms. This will enable interpretation of cerebral changes associated with the rehabilitation programme and functional impairments in NA. Discussion NA-CONTROL is the first randomised trial to investigate the effect of specific rehabilitation on residual complaints in NA. It also is the first study into the cerebral mechanisms that might underlie persistent scapular dyskinesia in NA. It thus may aid the further development of mechanism-based interventions for disturbed motor control in NA and in other peripheral neurological disorders. Trial registration ClinicalTrials.gov, NCT03441347. Registered on 20 February 2018. Electronic supplementary material The online version of this article (10.1186/s13063-019-3556-4) contains supplementary material, which is available to authorized users.

ObjectiveTo investigate the effectiveness of Energetic, a self-management group program combining aerobic training, energy conservation management, and relapse prevention to improve social participation in patients with neuromuscular disease (NMD) and chronic fatigue.MethodsIn this multicenter, assessor-blinded, 2-armed randomized controlled trial with repeated measurements, 53 patients with various types of NMD and chronic fatigue were randomly allocated to Energetic, a 4-month group intervention, or to usual care. The primary endpoint was social participation assessed with the Canadian Occupational Performance Measure (COPM) performance scale immediately postintervention. Secondary outcomes included COPM satisfaction scale, 6-Minute Walk Test (6MWT), and Checklist Individual Strength–subscale fatigue. Participants were followed for 11 months postintervention. Data were analyzed with linear models that account for repeated measurements.ResultsDirectly after intervention, the mean group difference for COPM-performance was 1.7 (95% confidence interval [CI] 1.0–2.4; p < 0.0001) in favor of the intervention group (n = 29), adjusted for baseline, sex, diagnosis, and work status. This effect was retained at 11 months follow-up (0.9; 95% CI 0.0–1.7; p = 0.049). The COPM satisfaction scale and 6MWT improved more in the intervention group compared to usual care. After 3 and 11 months follow-up, most beneficial effects on social participation and functional endurance were retained.ConclusionEnergetic led to sustainable improvements in social participation and functional endurance compared to usual care in patients with NMD and chronic fatigue.Clinicaltrials.gov identifierNCT02208687.Classification of evidenceThis study provides Class III evidence that a combination of aerobic training, energy conservation management, and relapse prevention improves social participation in patients with NMD and chronic fatigue.

BACKGROUND: Strength training or aerobic exercise programmes, or both, might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease. This is an update of a review first published in 2004 and last updated in 2013. We undertook an update to incorporate new evidence in this active area of research. OBJECTIVES: To assess the effects (benefits and harms) of strength training and aerobic exercise training in people with a muscle disease. SEARCH METHODS: We searched Cochrane Neuromuscular's Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL in November 2018 and clinical trials registries in December 2018. SELECTION CRITERIA: Randomised controlled trials (RCTs), quasi‐RCTs or cross‐over RCTs comparing strength or aerobic exercise training, or both lasting at least six weeks, to no training in people with a well‐described muscle disease diagnosis. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: We included 14 trials of aerobic exercise, strength training, or both, with an exercise duration of eight to 52 weeks, which included 428 participants with facioscapulohumeral muscular dystrophy (FSHD), dermatomyositis, polymyositis, mitochondrial myopathy, Duchenne muscular dystrophy (DMD), or myotonic dystrophy. Risk of bias was variable, as blinding of participants was not possible, some trials did not blind outcome assessors, and some did not use an intention‐to‐treat analysis. Strength training compared to no training (3 trials) For participants with FSHD (35 participants), there was low‐certainty evidence of little or no effect on dynamic strength of elbow flexors (MD 1.2 kgF, 95% CI −0.2 to 2.6), on isometric strength of elbow flexors (MD 0.5 kgF, 95% CI −0.7 to 1.8), and ankle dorsiflexors (MD 0.4 kgF, 95% CI −2.4 to 3.2), and on dynamic strength of ankle dorsiflexors (MD −0.4 kgF, 95% CI −2.3 to 1.4). For participants with myotonic dystrophy type 1 (35 participants), there was very low‐certainty evidence of a slight improvement in isometric wrist extensor strength (MD 8.0 N, 95% CI 0.7 to 15.3) and of little or no effect on hand grip force (MD 6.0 N, 95% CI −6.7 to 18.7), pinch grip force (MD 1.0 N, 95% CI −3.3 to 5.3) and isometric wrist flexor force (MD 7.0 N, 95% CI −3.4 to 17.4). Aerobic exercise training compared to no training (5 trials) For participants with DMD there was very low‐certainty evidence regarding the number of leg revolutions (MD 14.0, 95% CI −89.0 to 117.0; 23 participants) or arm revolutions (MD 34.8, 95% CI −68.2 to 137.8; 23 participants), during an assisted six‐minute cycle test, and very low‐certainty evidence regarding muscle strength (MD 1.7, 95% CI −1.9 to 5.3; 15 participants). For participants with FSHD, there was low‐certainty evidence of improvement in aerobic capacity (MD 1.1 L/min, 95% CI 0.4 to 1.8, 38 participants) and of little or no effect on knee extension strength (MD 0.1 kg, 95% CI −0.7 to 0.9, 52 participants). For participants with dermatomyositis and polymyositis (14 participants), there was very low‐certainty evidence regarding aerobic capacity (MD 14.6, 95% CI −1.0 to 30.2). Combined aerobic exercise and strength training compared to no training (6 trials) For participants with juvenile dermatomyositis (26 participants) there was low‐certainty evidence of an improvement in knee extensor strength on the right (MD 36.0 N, 95% CI 25.0 to 47.1) and left (MD 17 N 95% CI 0.5 to 33.5), but low‐certainty evidence of little or no effect on maximum force of hip flexors on the right (MD −9.0 N, 95% CI −22.4 to 4.4) or left (MD 6.0 N, 95% CI −6.6 to 18.6). This trial also provided low‐certainty evidence of a slight decrease of aerobic capacity (MD −1.2 min, 95% CI −1.6 to 0.9). For participants with dermatomyositis and polymyositis (21 participants), we found very low‐certainty evidence for slight increases in muscle strength as measured by dynamic strength of knee extensors on the right (MD 2.5 kg, 95% CI 1.8 to 3.3) and on the left (MD 2.7 kg, 95% CI 2.0 to 3.4) and no clear effect in isometric muscle strength of eight different muscles (MD 1.0, 95% CI −1.1 to 3.1). There was very low‐certainty evidence that there may be an increase in aerobic capacity, as measured with time to exhaustion in an incremental cycle test (17.5 min, 95% CI 8.0 to 27.0) and power performed at VO(2) max (maximal oxygen uptake) (18 W, 95% CI 15.0 to 21.0). For participants with mitochondrial myopathy (18 participants), we found very low‐certainty evidence regarding shoulder muscle (MD −5.0 kg, 95% CI −14.7 to 4.7), pectoralis major muscle (MD 6.4 kg, 95% CI −2.9 to 15.7), and anterior arm muscle strength (MD 7.3 kg, 95% CI −2.9 to 17.5). We found very low‐certainty evidence regarding aerobic capacity, as measured with mean time cycled (MD 23.7 min, 95% CI 2.6 to 44.8) and mean distance cycled until exhaustion (MD 9.7 km, 95% CI 1.5 to 17.9). One trial in myotonic dystrophy type 1 (35 participants) did not provide data on muscle strength or aerobic capacity following combined training. In this trial, muscle strength deteriorated in one person and one person had worse daytime sleepiness (very low‐certainty evidence). For participants with FSHD (16 participants), we found very low‐certainty evidence regarding muscle strength, aerobic capacity and VO(2) peak; the results were very imprecise. Most trials reported no adverse events other than muscle soreness or joint complaints (low‐ to very low‐certainty evidence). AUTHORS' CONCLUSIONS: The evidence regarding strength training and aerobic exercise interventions remains uncertain. Evidence suggests that strength training alone may have little or no effect, and that aerobic exercise training alone may lead to a possible improvement in aerobic capacity, but only for participants with FSHD. For combined aerobic exercise and strength training, there may be slight increases in muscle strength and aerobic capacity for people with dermatomyositis and polymyositis, and a slight decrease in aerobic capacity and increase in muscle strength for people with juvenile dermatomyositis. More research with robust methodology and greater numbers of participants is still required.

Background Gait impairments are common and disabling in chronic stroke patients. Pes equinovarus deformity is one of the primary motor deficits underlying reduced gait capacity after stroke. It predisposes to stance-phase instability and subsequent ankle sprain or falls. This instability is most pronounced when walking barefoot. Tarsal fusion is a recommended treatment option for varus deformity, but scientific evidence is sparse. We therefore evaluated whether a tarsal fusion improved barefoot walking capacity in chronic stroke patients with pes equinovarus deformity. Methods Ten patients with a pes equinovarus deformity secondary to supratentorial stroke underwent surgical correction involving a tarsal fusion of one or more joints. Instrumented gait analysis was performed pre- and postoperatively using a repeated-measures design. Primary outcome measure was gait speed. Results Walking speed significantly improved by 32% after surgery (0.38 m/s ± 0.20 to 0.50 m/s ± 0.17, p = 0.007). Significant improvement was also observed when looking at cadence (p = 0.028), stride length (p = 0.016), and paretic step length (p = 0.005). Step length on the nonparetic side did not change. Peak ankle moment increased significantly on the nonparetic side (p = 0.021), but not on the paretic side (p = 0.580). In addition, functional ambulation scores increased significantly (p = 0.008), as did satisfaction with gait performance (p = 0.017). Conclusions Tarsal fusion for equinovarus deformity in chronic stroke patients improves gait capacity, and the degree of improvement is of clinical relevance. Our results suggest that the improved gait capacity may be related to better prepositioning and loading of the paretic foot, leading to larger paretic step length and nonparetic ankle kinetics.

Objective: To evaluate construct validity and reproducibility of the Functional Gait Assessment (FGA) for measuring walking balance capacity in persons after stroke. Design: Cross-sectional study. Setting: Inpatient and outpatient rehabilitation center. Subjects: Fifty-two persons post-stroke (median (25% and 75% percentiles)) time post-stroke 6 (5–10) weeks) with independent walking ability (mean gait speed 1.1 ± .4 m/s). Methods: Subjects completed a standardized FGA twice within one to eight days by the same investigator. Validity was evaluated by testing hypotheses on the association with two timed walking tests, Berg Balance Scale, and the mobility domain of the Stroke Impact Scale using correlation coefficients ( r), and with Functional Ambulation Categories using the Kruskal–Wallis test. Reproducibility of FGA scores was assessed with intraclass correlation coefficient and standard error of measurement. Results: Subjects scored a median of 22 out of 30 points at the first FGA. Moderate to high significant correlations ( r .61–.83) and significant differences in FGA median scores between the Functional Ambulation Categories were found. Eight hypotheses (80%) could be confirmed. Inter-rater, intra-rater, and test–retest reliability of the total scores were excellent. The standard error of measurement and minimal detectable change were 2 and 6 points, respectively. No relevant ceiling effect was observed. Conclusion: The FGA demonstrated good measurement properties in persons after stroke and yielded no ceiling effect in contrast to other capacity measures. In clinical practice, a measurement error of 6 points should be taken into account in interpreting changes in walking balance.

Objective Toe walking due to progressive shortening of the calf muscles is common in people with hereditary spastic paraplegia. Achilles tendon lengthening is a treatment option, but clinicians are often hesitant to use this procedure, as it may result in weakening of the calf muscles and, subsequently, in reduced ankle power and knee instability during the stance phase of gait. We report here a case report supporting that these negative side-effects can be avoided in well-selected people with hereditary spastic paraplegia. Method Bilateral Achilles tendon lengthening, combined with bilateral tenotomy of the tibialis posterior and toe flexors, was performed in a 29-year-old woman with uncomplicated hereditary spastic paraplegia who experienced progressive gait instability due to shortening of the soleus and gastrocnemius muscles (resulting in irreducible pes equinus). Results Bilateral Achilles tendon lengthening resulted in improvement in both subjective and objective outcomes. Self-selected gait speed improved from 0.75 m/s before surgery to 1.07 m/s after surgery (p < 0.001). Knee instability during the stance phase did not occur post-surgery. The ankle moment trajectories normalized after surgery, while peak ankle powers increased. Conclusion Correction of bilateral irreducible pes equinus by Achilles tendon lengthening may improve gait capacity in well-selected subjects with hereditary spastic paraplegia. LAY ABSTRACT Toe walking due to shortening of the calf muscles is common in people with hereditary spastic paraplegia. This case report supports that lengthening of the calf muscles (Achilles tendon lengthening) may improve gait in well-selected people with hereditary spastic paraplegia.

Contains fulltext : 173234.pdf (Publisher’s version ) (Closed access) Purpose To investigate the upper extremity (UE) at the level of impairments and related activity limitations and participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). Methods The study was conducted using web-based questionnaires that were distributed amongst people with FSHD in the Netherlands. Eighty-eight respondents started the survey, and 71 completed it. The questionnaires covered the following dimensions: Function, Activity and Participation of the International Classification of Functioning Disability and Health. Results More than 40% of the respondents experienced pain in one arm or both the arms. Increased pain and stiffness scores and longer disease duration were associated with increased limitation scores. For basic activities, lifting the arm above shoulder-level was most frequently reported as most limited, coherent with the clinical picture of FSHD. Among the respondents, 50% indicated restrictions at school, 78% indicated restrictions at work and more than 80% indicated restrictions whilst participating in sports, hobbies, household activities and romantic relationships. Conclusions This study has shown that alongside the well-known problem of lifting the arms above shoulder-level, UE activities below shoulder height during vocational and occupational activities are also problematic in patients with FSHD. Alongside disease duration, pain and stiffness are associated with UE activity limitations. Implications for Rehabilitation Attention is needed for pain and experienced stiffness in the upper extremity as it is frequently present in patients with FSHD. Rehabilitation professionals need to be aware that patients with FSHD not only experience problems with activities above shoulder height, but also with activities below shoulder height. At least 50% of the patients with FSHD experience restrictions in participation as a result of limitations in their UE.

Aim To systematically review the efficacy of interventions on upper limb function in children 0 to 19 years of age with bilateral cerebral palsy on the basis of outcome measures of upper limb function and measures of activities and/or participation according to the International Classification of Functioning, Disability and Health. Method Cochrane, PubMed, Embase, CINAHL, and Web of Science were searched from inception to September 2017. Methodological quality and strength of evidence were analysed by two independent raters using Sackett's level of evidence and the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) guidelines. Results Fifteen studies with a large variety of interventions and heterogeneity in outcome measures met the inclusion criteria. Twelve studies provided level IV evidence according to AACPDM guidelines. For three small randomized controlled trials the level of evidence was II. Only one of these trials showed strong methodological quality: a study on hand–arm bimanual intensive therapy including lower extremities. Interpretation We identified a large variety of interventions, heterogeneity in outcome measures, and generally weak to moderate methodological quality for most studies. We recommend further research specifically aimed at bimanual‐intensive, goal‐directed, and task‐specific training programmes for the upper limb in children with bilateral cerebral palsy, using either high‐quality (multicentre) trials or well‐designed single‐case trials. What this paper adds There is a large variety of interventions on upper limb function in children with bilateral cerebral palsy.Heterogeneity of outcome measures and interventions impeded firm conclusions about intervention efficacy.Most studies had low‐level evidence and weak to moderate methodological quality.The strongest evidence from a small randomized controlled trial was for hand–arm bimanual intensive therapy including lower extremities., What this paper adds There is a large variety of interventions on upper limb function in children with bilateral cerebral palsy.Heterogeneity of outcome measures and interventions impeded firm conclusions about intervention efficacy.Most studies had low‐level evidence and weak to moderate methodological quality.The strongest evidence from a small randomized controlled trial was for hand–arm bimanual intensive therapy including lower extremities. This article's abstract has been translated into Spanish and Portuguese. Follow the links from the http://onlinelibrary.wiley.com/doi/10.1111/dmcn.14141/abstract to view the translations.

Objective: To investigate construct validity and test–retest reliability of the parent-rated Hand-Use-at-Home questionnaire (HUH) in children with neonatal brachial plexus palsy or unilateral cerebral palsy. Design and subjects: For this cross-sectional study, children with neonatal brachial plexus palsy or unilateral cerebral palsy, aged 3–10 years, were eligible. Main measures: The HUH, Pediatric Outcome Data Collection Instrument Upper Extremity Scale (neonatal brachial plexus palsy only), and Children’s Hand-Use Experience Questionnaire (unilateral cerebral palsy only) were completed. The HUH was completed twice in subgroups of both diagnoses. Lesion-extent (indication of involved nerve rootlets in neonatal brachial plexus palsy as confirmed during clinical observation and/or nerve surgery) and Manual Ability Classification System levels (unilateral cerebral palsy) were obtained from the medical records. Spearman correlation coefficients between the HUH and all clinical variables, agreement, standard error of measurement, smallest detectable change and intra-class correlation were calculated. Results: A total of 260 patients participated (neonatal brachial plexus palsy: 181), of which 56 completed the second HUH (neonatal brachial plexus palsy: 16). Median age was 6.9 years for children with neonatal brachial plexus palsy, 116 had C5-C6 lesions. Median age for children with unilateral cerebral palsy was 6.4 years, 33 had Manual Ability Classification System Level II. The HUH correlated moderately with lesion-extent ( rs =−0.5), Pediatric Outcome Data Collection Instrument Upper Extremity Scale ( rs = 0.6) and Children’s Hand-Use Experience Questionnaire ( rs = 0.5) but weakly with Manual Ability Classification System levels ( rs = −0.4). Test–retest reliability was excellent (intra-class correlation2,1 = 0.89, standard error of measurement = 0.599 and smallest detectable change = 1.66 logits) and agreement was good (mean difference HUH1 − HUH2 = 0.06 logits). Conclusion: The HUH showed good construct validity and test–retest reliability in children with neonatal brachial plexus palsy or unilateral cerebral palsy.

Purpose To assess the interrater reliability, construct validity and usability of the Eating and Drinking Ability Classification System (EDACS) among Dutch children with Cerebral Palsy (CP) when used by speech and language therapists (SLTs) familiar and unfamiliar with the child's eating and drinking performance and parents. Methods Translation was undertaken using the method of Eremenco. Agreement between SLTs and parents when using EDACS was determined by intraclass correlation coefficient (ICC) and linear weighted Kappa (κW). Associations with other functional classification systems including the Dysphagia Management Staging Scale (DMSS) were investigated to determine construct validity by Kendall's tau-b. Results Thirty-one SLTs classified 149 children (67 girls; mean 10 y, SD 4 y, range 3-21 y) with EDACS. Pairs of SLTs showed good agreement ([ICC] = 0.84; 95% confidence interval [CI] 0.79-0.88; [κW] = 0.71). Eighty-one parents showed good agreement with SLTs (n= 31) as well (ICC = 0.80; 95% CI 0.71-0.87; κW= 0.61). There was a significant and strong positive correlation of EDACS with DMSS (Kendall's tau-b 0.81) supporting its construct validity. Usability of EDACS was generally good. Conclusion The Dutch version of EDACS is reliable and valid, and can be used easily by (familiar and unfamiliar) SLTs and parents of children with CP. Parents and professionals showed a high level of consistency when classifying eating and drinking abilities. EDACS enables uniform and efficient communication about safety and efficiency of functional eating and drinking ability in clinical and research contexts.

Drop foot is a common problem following neurological conditions such as stroke, multiple sclerosis (MS), traumatic brain injury (TBI), incomplete spinal cord injury (iSCI) and cerebral palsy (CP). Between 20 and 30% of patients entering neurological rehabilitation suffer from drop foot. Typically, drop foot is caused by weakness of the ankle dorsiflexors leading to a lack of foot elevation during the swing phase of gait, which is often accompanied by a tendency towards varus deviation at the ankle due to muscular imbalance. In addition, spasticity of the ankle plantarflexors may worsen equinovarus deviation as a result of muscle stiffness, contracture and pathological co-contraction. Drop foot leads to an abnormal gait pattern, decreased walking speed, limited endurance walking and increased fall risk. All these factors can limit mobility, independence and social participation leading to reduced quality of life.

Many patients with incomplete spinal cord injury (iSCI) have impaired gait and balance capacity, which may impact daily functioning. Reduced walking speed and impaired gait stability are considered important underlying factors for reduced daily functioning. With conventional therapy, patients are limited in training gait stability, but this can be trained on a treadmill in a virtual environment, such as with the Gait Real-time Analysis Interactive Lab (GRAIL). Our objective was to evaluate the effect of 6-weeks GRAIL-training on gait and dynamic balance in ambulatory iSCI patients. In addition, the long-term effect was assessed. Fifteen patients with chronic iSCI participated. The GRAIL training consisted of 12 one-hour training sessions during a 6-week period. Patients performed 2 minute walking tests on the GRAIL in a self-paced mode at the 2nd, and 3rd (baseline measurements) and at the 12th training session. Ten patients performed an additional measurement after 6 months. The primary outcome was walking speed. Secondary outcomes were stride length, stride frequency, step width, and balance confidence. In addition, biomechanical gait stability measures based on the position of the center of mass (CoM) or the extrapolated center of mass (XCoM) relative to the center of pressure (CoP) or the base of support (BoS) were derived: dynamic stability margin (DSM), XCoM-CoP distance in anterior-posterior (AP) and medial-lateral (ML) directions, and CoM-CoP inclination angles in AP and ML directions. The effect of GRAIL-training was tested with a one-way repeated measures ANOVA (α = 0.05) and post-hoc paired samples t-tests (α = 0.017). Walking speed was higher after GRAIL training (1.04 m/s) compared to both baseline measurements (0.85 and 0.93 m/s) (p < 0.001). Significant improvements were also found for stride length (p < 0.001) and stability measures in AP direction (XCoM-CoPAP (p < 0.001) and CoM-CoPAP−angle (p < 0.001)). Stride frequency (p = 0.27), step width (p = 0.19), and stability measures DSM (p = 0.06), XCoM-CoPML (p = 0.97), and CoM-CoPML−angle (p = 0.69) did not improve. Balance confidence was increased after GRAIL training (p = 0.001). The effects were remained at 6 months. Increased walking speed, stride length, AP gait stability, and balance confidence suggest that GRAIL-training improves gait and dynamic balance in patients with chronic iSCI. In contrast, stability measures in ML direction did not respond to GRAIL-training.

Background. The relationships between motor impairment of the affected leg, postural control asymmetry, and impaired body sway control after stroke are not well understood. Objective. To examine the relationship between motor impairment of the affected leg and reduced contribution of this leg to body sway control (ie, dynamic control asymmetry [DCA]) and to determine the relationships between impaired body sway control, DCA, and weight-bearing asymmetry (WBA). Methods. We assessed quiet-standing balance with eyes open in 70 persons with a unilateral supratentorial chronic stroke using 2 force plates. Center-of-pressure (COP) velocity was calculated for both feet together in the anteroposterior (AP) and mediolateral (ML) directions as a measure of body sway control. Bilateral AP COP velocities were used to calculate an index for DCA and weight borne on each side to calculate WBA. Fugl-Meyer assessment of the lower extremity (FMA-LE; range: 0-28) served as a measure of affected-leg motor impairment. Results. All participants with FMA-LE s = 0.496; P < .001), and less ML sway control ( rs = 0.268; P = .025). AP sway control was not significantly related to either DCA or WBA. Conclusions. Even clinically well-recovered stroke survivors with (near) maximal FMA-LE scores may show clear postural asymmetry in terms of the relative contribution of the affected leg to body sway control. WBA seems to be an effective compensatory mechanism to optimize the contribution of the less-affected leg to balance, particularly in the AP direction.

Item does not contain fulltext OBJECTIVE: To evaluate the construct validity and interrater reliability of 4 simple antigravity tests in a small group of patients with facioscapulohumeral muscular dystrophy (FSHD). DESIGN: Case-control study. SETTING: University medical center. PARTICIPANTS: Patients with various severity levels of FSHD (n=9) and healthy control subjects (n=10) were included (N=19). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: A 4-point ordinal scale was designed to grade performance on the following 4 antigravity tests: sit to stance, stance to sit, step up, and step down. In addition, the 6-minute walk test, 10-m walking test, Berg Balance Scale, and timed Up and Go test were administered as conventional tests. Construct validity was determined by linear regression analysis using the Clinical Severity Score (CSS) as the dependent variable. Interrater agreement was tested using a kappa analysis. RESULTS: Patients with FSHD performed worse on all 4 antigravity tests compared with the controls. Stronger correlations were found within than between test categories (antigravity vs conventional). The antigravity tests revealed the highest explained variance with regard to the CSS (R(2)=.86, P=.014). Interrater agreement was generally good. CONCLUSIONS: The results of this exploratory study support the construct validity and interrater reliability of the proposed antigravity tests for the assessment of functional capacity in patients with FSHD taking into account the use of compensatory strategies. Future research should further validate these results in a larger sample of patients with FSHD.

Contains fulltext : 153108.pdf (Publisher’s version ) (Closed access) After stroke, the ability to make step adjustments during walking is reduced and requires more attention, which may cause problems during community walking. The C-Mill is an innovative treadmill augmented with visual context (e.g., obstacles and stepping targets), which was designed specifically to practice gait adaptability. The objective of this study was to determine whether C-Mill gait adaptability training can help to improve gait adjustments and associated attentional demands. Sixteen community-ambulating persons in the chronic stage of stroke (age: 54.8 +/- 10.8 years) received ten sessions of C-Mill training within 5-6 weeks. Prior to and after the intervention period, participants performed an obstacle-avoidance task with and without a secondary attention-demanding auditory Stroop task to assess their ability to make gait adjustments (i.e., obstacle-avoidance success rates) as well as the associated attentional demands (i.e., Stroop success rates, stratified for pre-crossing, crossing, and post-crossing strides). Obstacle-avoidance success rates improved after C-Mill training from 52.4 +/- 16.3 % at pretest to 77.0 +/- 16.4 % at posttest (p < 0.001). This improvement was accompanied by greater Stroop success rates during the obstacle-crossing stride only (pretest: 62.9 +/- 24.9 %, posttest: 77.5 +/- 20.4 %, p = 0.006). The observed improvements in obstacle-avoidance success rates and Stroop success rates were strongly correlated (r = 0.68, p = 0.015). The ability to make gait adjustments and the associated attentional demands can be successfully targeted in persons with stroke using C-Mill training, which suggests that its underlying assumptions regarding motor control are appropriate. This study lends support and guidance for designing a randomized controlled trial to further examine the potential of C-Mill training for improving safe community ambulation after stroke.

Contains fulltext : 155319.pdf (Publisher’s version ) (Open Access) OBJECTIVE: Although calf muscle spasticity is often treated with botulinum toxin type-A, the effects on balance and gait are ambiguous. Hereditary spastic paraplegia is characterized by progressive spasticity and relatively mild muscle weakness of the lower limbs. It is therefore a good model to evaluate the functional effects of botulinum toxin type-A. DESIGN: Explorative pre-post intervention study. SUBJECTS: Fifteen subjects with pure hereditary spastic paraplegia. METHODS: Patients with symptomatic calf muscle spasticity and preserved calf muscle strength received botulinum toxin type-A injections in each triceps surae (Dysport(R), 500-750 MU) followed by daily stretching exercises (18 weeks). Before intervention (T0), and 4 (T1) and 18 (T2) weeks thereafter, gait, balance, motor selectivity, calf muscle tone and strength were tested. RESULTS: Mean comfortable gait velocity increased from T0 (0.90 m/s (standard deviation (SD) 0.18)) to T1 (0.98 m/s (SD 0.20)), which effect persisted at T2, whereas balance and other functional measures remained unchanged. Calf muscle tone declined from T0 (median 2; range 1-2) to T1 (median 0; range 0-1), which effect partially persisted at T2 (median 1; range 0-2). Calf muscle strength did not change. CONCLUSION: Botulinum toxin type-A treatment and subsequent muscle stretching of the calves improved comfortable gait velocity and reduced muscle tone in patients with hereditary spastic paraplegia, while preserving muscle strength. Balance remained unaffected.

Contains fulltext : 176980.pdf (Publisher’s version ) (Open Access) BACKGROUND: Cognitive behavioural therapy does not reduce fatigue in post-polio syndrome, but is effective in facioscapulohumeral dystrophy. This difference in efficacy might be explained by a different role of cognitions in these conditions. OBJECTIVE: To compare fatigue-related cognitions between patients with post-polio syndrome and facio-scapulohumeral dystrophy. SUBJECTS: Patients with post-polio syndrome (n = 21) and facioscapulohumeral dystrophy (n = 24) allocated to a cognitive behavioural therapy intervention in 2 identical trials. METHODS: Assessed cognitions included: sense of control over fatigue; catastrophizing; acceptance; focusing on fatigue; and perceived social support. Group differences in cognitions (independent t-tests or Mann-Whitney U tests) and group differences in the association of cognitions with fatigue (linear regression models) were studied. RESULTS: No differences in cognitions were found between the 2 groups (p > 0.18). Furthermore, there were no cognition-by-group interaction effects, except for "perceived social support", for which a different association with fatigue was found between the 2 groups (p = 0.01). However, univariate models revealed no associations per group. CONCLUSION: Fatigue-related cognitions in severely fatigued patients with post-polio syndrome are not clearly different from that in facioscapulohumeral dystrophy. Thus, the lack of efficacy of cognitive behavioural therapy in post-polio syndrome cannot be attributed to unique cognitive characteristics of this population.

Contains fulltext : 175692.pdf (Publisher’s version ) (Open Access) Aim: To describe the development of the parent-rated Hand-Use-at-Home questionnaire (HUH) assessing the amount of spontaneous use of the affected hand in children with unilateral paresis, and to test its internal structure, unidimensionality, and validity. Method: Parents of children with unilateral cerebral palsy (CP) and professionals participated in the development of the HUH. To examine internal validity, data of 322 children (158 males, 164 females; mean age 6y 7mo, standard deviation [SD] 2y 1mo) with unilateral CP (n=131) or neonatal brachial plexus palsy (NBPP) (n=191) were collected. Rasch analysis was used to examine discriminative capacity of the 5-category rating scale as well as unidimensionality and hierarchy of the item set. Additionally, data of 55 children with typical development (24 males, 31 females; 6y 9mo, SD 2y 5mo) were used to examine construct validity. Results: The 5-category rating scale was disordered in all items and was collapsed to obtain the best discriminating sum score. Ten misfitting or redundant items were removed. Eighteen hierarchically ordered bimanual items fitted the unidimensional model within acceptable range. The HUH significantly discriminated between the three groups (children with typical development, NBPP, unilateral CP; H(2)=118.985, p

Contains fulltext : 174287.pdf (Publisher’s version ) (Open Access) AIM: The aims of this study were: (1) to determine the incidence and nature of adverse effects on oral motor function after first injections of botulinum neurotoxin A (BoNT-A) in submandibular glands for excessive drooling in children with central nervous system disorders; and (2) to identify independent predictors of these adverse effects. METHOD: A cohort study involved 209 children (123 males, 86 females, aged 4-27y, median 8y 4mo), who received submandibular BoNT-A injections for drooling. Adverse effects were categorized into swallowing, eating, drinking, articulation, and other problems. Univariable logistic regression was used to study differences in patients with and without adverse effects. Possible predictors were identified using multivariable logistic regression. RESULTS: Transient adverse effects occurred in 33% of the 209 BoNT-A treatments. Almost 80% of these were mild, versus 8.7% severe. Approximately 54% of the adverse effects spontaneously resolved within 4 weeks; 3% still existed after 32 weeks. A diagnosis of cerebral palsy, higher range of BoNT-A dosage, and a pre-treatment drooling quotient

Item does not contain fulltext To better understand postural and movement disabilities, the pattern of total body muscle fat infiltration was analyzed in a large group of patients with facioscapulohumeral muscular dystrophy. Additionally, we studied whether residual D4Z4 repeat array length adjusted for age and gender could predict the degree of muscle involvement. Total body computed tomography scans of 70 patients were used to assess the degree of fat infiltration of 42 muscles from neck to ankle level on a semi-quantitative scale. Groups of muscles that highly correlated regarding fat infiltration were identified using factor analysis. Linear regression analysis was performed using muscle fat infiltration as the dependent variable and D4Z4 repeat length and age as independent variables. A pattern of muscle fat infiltration in facioscapulohumeral muscular dystrophy could be constructed. Trunk muscles were most frequently affected. Of these, back extensors were more frequently affected than previously reported. Asymmetry in muscle involvement was seen in 45% of the muscles that were infiltrated with fat. The right-sided upper extremity showed significantly higher scores for fat infiltration compared to the left side, which could not be explained by handedness. It was possible to explain 29% of the fat infiltration based on D4Z4 repeat length, corrected for age and gender. Based on our results we conclude that frequent involvement of fat infiltration in back extensors, in addition to the abdominal muscles, emphasizes the extent of trunk involvement, which may have a profound impact on postural control even in otherwise mildly affected patients.